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Rabbit Anti-Phospho-HER3 (Tyr1276)/FITC Conjugated antibody
background:
ErbB3 is a member of the epidermal growth factor receptor (EGFR) family of receptor tyrosine kinases. ErbB3 is a membrane-bound protein which has a neuregulin binding domain but not an active kinase domain. It can therefore bind this ligand but cannot convey a signal into the cell via protein phosphorylation. However it does form heterodimers with other EGF receptor family members which do have kinase activity. Heterodimerization leads to the activation of pathways which lead to cell proliferation or differentiation. Amplification of this gene and/or overexpression of its protein have been reported in numerous cancers including prostate, bladder and breast tumors. Alternate transcriptional splice variants encoding different isoforms have been characterized. Isoform 2 lacks the intermembrane region and is secreted outside the cell. This form acts to modulate the activity of the membrane-bound form. Additional splice variants have also been reported but they have not been thoroughly characterized. Defects in ERBB3 are the cause of lethal congenital contracture syndrome type 2 (LCCS2); also called Israeli Bedouin multiple contracture syndrome type A. LCCS2 is an autosomal recessive neurogenic form of a neonatally lethal arthrogryposis that is associated with atrophy of the anterior horn of the spinal cord.
Function:
Binds and is activated by neuregulins and NTAK.
Subunit:
Monomer and homodimer. Heterodimer with each of the other ERBB receptors (Potential). Interacts with CSPG5, PA2G4, GRB7 and MUC1.
Subcellular Location:
Isoform 1: Cell membrane; Single-pass type I. membrane protein. Isoform 2: Secreted.
Tissue Specificity:
Epithelial tissues and brain.
Post-translational modifications:
Ligand-binding increases phosphorylation on tyrosine residues and promotes its association with the p85 subunit of phosphatidylinositol 3-kinase. Subject to autophosphorylation.
DISEASE:
Defects in ERBB3 are the cause of lethal congenital contracture syndrome type 2 (LCCS2) [MIM:607598]; also called Israeli Bedouin multiple contracture syndrome type A. LCCS2 is an autosomal recessive neurogenic form of a neonatally lethal arthrogryposis that is associated with atrophy of the anterior horn of the spinal cord. The LCCS2 syndrome is characterized by multiple joint contractures, anterior horn atrophy in the spinal cord, and a unique feature of a markedly distended urinary bladder. The phenotype suggests a spinal cord neuropathic etiology.
Similarity:
Belongs to the protein kinase superfamily.
Tyr protein kinase family.
EGF receptor subfamily.
Contains 1 protein kinase domain.
Database links:
Entrez Gene: 2065 Human
Entrez Gene: 13867 Mouse
Entrez Gene: 29496 Rat
Omim: 190151 Human
SwissProt: P21860 Human
SwissProt: Q61526 Mouse
SwissProt: Q62799 Rat
Unigene: 118681 Human
Unigene: 373043 Mouse
Unigene: 10228 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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